Nineteen patients with types I and III Ehlers-Danlos syndrome were hospitalized at our institution between 1973 and 1978. Chest roentgenogram, electrocardiogram, and echocardiogram were done; 11 patients underwent cardiac catheterization. Thirty-five cardiac or great vessel abnormalities were detected. Fifteen patients had nitral valve prolapse; six also had tricuspid valve prolapse. Dilatation of the aortic root or extasia of the sinuses of Valsalva, or both, occurred in six patients. Dilatation of the pulmonary artery and annulus caused pulmonary regurgitation in one patient. Congenital heart defects included bicuspid aortic valve (two), pulmonary valvular stenosis (one), ventricular septal defect (two), and an atrial septal defect (one). The apparent high prevalence of cardiovascular abnormalities in hospitalized patients with types I and III Ehlers-Danlos syndrome necessitates a careful cardiovascular evaluation. Conversely, Ehlers-Danlos syndrome types I and III should be excluded in patients with mitral or tricuspid valve prolapse, great vessel dilatation, and congenital heart defects.

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A patient is described with acute pancreatitis which was probably caused by furosemide. Administration of furosemide on two separate occasions was associated with increases in serum amylase concentrations and recurrence of abdominal pain. This case is of further interest because of the presence of hyperlipemia in the absence of an underlying lipid abnormality. Following recovery from pancreatitis, the lipoprotein pattern evolved from type V to type III, type IIA, and finally to normal.

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A study of neuro-endocrine and behavioral response to psychological stress of 11 children having heart surgery was initiated. An attempt was made to learn if more overtly anxious children would respond differently during hospitalization and surgery than those who appeared to have better emotional control. All children in this study survived the surgery. It is suggested that prognosis is greatly related to the coping capacity of a child and his family to stress; that prognoses can be predicted if adequate assessments can be made; and that as more precise methods for evaluation of the neuro-endocrine response become available it will be useful to employ them to explore the complicated relationship between the central nervous system and the endocrine organs in relation to children under stress. Assessment of anxiety in affect and function was made by a child psychiatrist and a pediatric nurse specialist during the hospitalization and at outpatient follow-up. Twenty-four-hour urine collections were made after suture removal and pre- and post-discharge and analyzed for 17-hydroxycorticosteroids by a modification of the method of Porter and Silber. In terms of emotional stimulation of the adrenocortical axis, the days before surgery and of return from intensive care were the most stressful of the days studied. The mean value on the day before surgery was significantly greater than that on the day before discharge. Values for the day before and after suture removal were also elevated in comparison with the day before discharge. Hospitalization was seen as stressful in terms of the parameter studied with the mean of all inpatient values including the day of discharge being approximately double that obtained by pooling all outpatient determinations. Surprising to the researchers was the fact that no difference was found between values for overtly excessively anxious children versus their less anxious counterparts on specific days, or when pooled in-hospital values were contrasted. The degree of elevation of 17-OHCS was related to the situation. No correlation between our estimate of anxiety and 17-OHCS levels was found.